Type I interferonopathies represent an emerging group of autoinflammatory disorders, with clinical characteristics and therapeutic response very different from previously characterized “inflammasome diseases.” Pediatricians must be aware of the clinical “IFN alarm” signals (such as chilblain-like vasculitis and interstitial lung disease) in order to suspect them and to direct properly the diagnostic workup with eminent prognostic implications. This evidence concerns the gene IFNA1 and vasculitis.